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Proc (Bayl Univ Med Cent). 2014 Jan;27(1):22-4.

Systemic mastocytosis with associated acute myelogenous leukemia.

Author information

  • Department of Hematology/Oncology (Zhrebker, Cooper) and the Department of Pathology, Section of Hematopathology (Krause), Baylor University Medical Center at Dallas and the Baylor Charles A. Sammons Cancer Center at Dallas.


Systemic mastocytosis (SM) is a condition associated with a clonal neoplastic proliferation of mast cells. Approximately 40% of patients with SM present with an associated clonal hematological non-mast cell lineage disorder. Patients presenting with SM-acute myeloid leukemia (AML) have the worst prognosis. We present a case of a 62-year-old woman who was diagnosed with SM-AML. After initial treatment with a standard regimen of cytosine arabinoside (Ara-C)/idarubicin, her bone marrow showed residual blasts. She was subsequently treated with a second induction regimen of clofarabine and high-dose Ara-C, which resulted in remission of AML, although a residual mast cell infiltrate persisted in her bone marrow. After consolidation therapy with clofarabine/Ara-C, the patient received a stem cell allograft. A follow-up bone marrow showed no residual blasts but persistent mast cells occupying about 5% of the marrow volume.

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